Abstract: AIM:To analyze the causes of acute lung injury (ALI) after surgical treatment of cyanotic congenital heart disease (C-CHD) and to summarize the postoperative therapeutic experience. METHODS: Included in the study were 79 patients with C-CHD who underwent surgical treatment in our hospital from March 2007 to October 2011; 12 patients developed ALI postoperatively. Transcutaneous oxygen saturation (SpO2), hematocrit (HCT), McGoon ratio, Nakata index and CPB data and therapeutic data of the 12 patients with ALI were collected. RESULTS: There were 12 cases complicated by ALI with a morbidity of 15%. The mean age of the 12 patients was (19±8) months (8-63 months) and the mean body weight was (15±4) kg (8-25 kg). Surgical procedures of the 12 cases were all performed under intravenous-inhalation combined anesthesia and cardiopulmonary bypass (CPB) and modified ultrafiltration was used postoperatively. Total CPB time and aortic clamp time were (117±12) min and (81±13) min, respectively. Peritoneal dialysis (PD) was performed in 11 cases. Five patients were given inhaled nitric oxide (NO) and one patient was given pulmonary surfactant (PS). Four patients died and the total mortality rate was 33.3%. CONCLUSION: Incidence of ALI after surgical treatment of C-CHD is high. Proper mechanical ventilation and appropriate fluid balance will improve pulmonary function as well as early recovery of the patients. A positive PS replacement or NO inhalation therapy may be useful.